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Hypoplastic left heart syndrome

Takeshi Konuma, Syunsuke Sakamoto, Syuhei Toba, Ayano Futsuki, Naoki Yamamoto, Shinji Kanemitsu, Hideto Shimpo
OBJECTIVES: Postoperative left pulmonary artery (PA) or bronchus compression occasionally follows narrowing of the retroaortic space after the Norwood procedure. We examined hypoplastic left heart syndrome (HLHS)-specific anatomical malformations and hypothesized that a long main pulmonary arterial trunk might contribute to a larger retroaortic space. In this study, we introduced a modified Norwood procedure [pulmonary artery trunk-saving method (PATS)] and evaluated the results. METHODS: HLHS-specific anatomical malformations were examined using computed tomographic data from 23 consecutive patients who underwent bilateral pulmonary banding...
March 12, 2018: Interactive Cardiovascular and Thoracic Surgery
Jacob Y Cao, Seung Yeon Lee, Kevin Phan, Julian Ayer, David S Celermajer, David S Winlaw
The hybrid strategy is an alternative to the traditional Norwood procedure for initial palliation of infants with hypoplastic left heart syndrome (HLHS) who are deemed to be at high surgical risk. Numerous single-center studies have compared the two procedures, showing similar early outcomes, although the cohort sizes are likely insufficiently powered to detect significant differences. The current meta-analysis aims to explore the early morbidity and mortality associated with the hybrid compared to the Norwood procedure...
March 2018: World Journal for Pediatric & Congenital Heart Surgery
Qinghai Peng, Shi Zeng, Qichang Zhou, Wen Deng, Tao Wang, Ya Tan, Yushan Liu
To observe Doppler changes in the three main cerebral arteries in fetuses with congenital heart defects (CHDs). The pulsatility index (PI) values of the anterior cerebral artery (ACA), middle cerebral artery (MCA), and posterior cerebral artery (PCA) were prospectively compared in 78 CHD fetuses and 78 normal control fetuses. Correlations between the cerebral artery PIs and the neurodevelopment scores (psychomotor development index [PDI] and mental development index [MDI]) were assessed. The MCA-PI was decreased significantly in fetuses with hypoplastic left heart syndrome (HLHS)...
March 14, 2018: Scientific Reports
Peter Murin, Nicodème Sinzobahamvya, Antonia Schulz, Victoria Lorenzen, Stanislav Ovroutski, Felix Berger, Joachim Photiadis, Mi-Young Cho
OBJECTIVES: The technique of subcoronary autograft implantation for the Ross procedure has shown excellent durability of aortic valve function in adults. However, its use in children with hypoplastic aortic annulus or multilevel left ventricular outflow tract obstruction (LVOTO) was traditionally precluded. We combined this technique with a Konno incision and evaluated LVOTO relief and durability of the autograft function in growing patients. METHODS: Between January 2012 and January 2017, 13 patients with severe LVOTO and hypoplastic aortic annulus underwent Ross-Konno procedure with subcoronary autograft implantation...
March 9, 2018: Interactive Cardiovascular and Thoracic Surgery
Patricio E Lau, Stephanie M Cruz, Elena C Ocampo, Sushma Nuthakki, Candace C Style, Timothy C Lee, David E Wesson, Oluyinka O Olutoye
PURPOSE: The purpose of this study was to evaluate the characteristics of patients with congenital heart disease (CHD) who developed necrotizing enterocolitis (NEC). METHODS: A retrospective review of neonates with CHD at a tertiary care center between January 2006 and January 2016 was performed. Diagnosis of NEC was based on modified Bell's criteria. Patients were grouped by Risk Adjustment for Congenital Heart Surgery (RACHS-1) or by ductal-dependent (DD) lesions that require a patent ductus arteriosus to supply pulmonary or systemic circulation...
February 7, 2018: Journal of Pediatric Surgery
Natasha L Lopez, Charitha Gowda, Carl H Backes, Deipanjan Nandi, Holly Miller-Tate, Samantha Fichtner, Robin Allen, Jamie Stewart, Clifford L Cua
INTRODUCTION: Neonates with hypoplastic left heart syndrome (HLHS) are at increased risk for necrotizing enterocolitis (NEC). Initial hospital outcomes are well described, but minimal midterm data exist. Goal of this study was to compare outcomes of HLHS infants with NEC (HLHS-NEC) to HLHS without NEC (HLHS-nNEC) during the interstage period. METHODS: Data were reviewed from 55 centers using the NPC-QIC database. Case-control study with one HLHS-NEC matched to HLHS-nNEC neonates in a 1:3 ratio based on institutional site, type of surgical repair, and gestational age ±1 week was performed...
March 9, 2018: Congenital Heart Disease
Samuel Wittekind, Wayne Mays, Yvette Gerdes, Sandra Knecht, John Hambrook, William Border, John Lynn Jefferies
Patients with a Fontan circulation have impaired exercise capacity. Cardiac rehabilitation (CR) has shown promise in enhancing peak exercise parameters in this population, but an improvement in submaximal exercise has not been consistently demonstrated. We assessed the hypothesis that participation in CR will be associated with more efficient oxygen extraction and ventilation during submaximal exercise. In this prospective study, pediatric Fontans completed two 60 min CR sessions per week for 12 weeks. Cardiopulmonary exercise testing and stress echocardiography were performed at baseline and last CR session, and then compared with a paired sample t test...
March 9, 2018: Pediatric Cardiology
Takashi Kido, Takaya Hoashi, Masataka Kitano, Masatoshi Shimada, Kenichi Kurosaki, Hatsue Ishibashi-Ueda, Hajime Ichikawa
The purpose of the study is to analyze the impact of hybrid stage 1 palliation on right ventricular myocardial pathology in hypoplastic left heart syndrome. Sufficient amount of right ventricular biopsies could be obtained from 16 of 32 patients who underwent Norwood operation between 2007 and 2013. Histopathological findings of right ventricle in patients who underwent primary Norwood operation (primary group, n = 5), patients with aortic atresia (HS1P AA group, n = 6) or aortic stenosis (HS1P AS group, n = 5) who underwent staged Norwood palliation following hybrid stage 1 palliation were compared...
March 9, 2018: Pediatric Cardiology
Shaji C Menon, Ragheed Al-Dulaimi, Brian W McCrindle, David J Goldberg, Ritu Sachdeva, Bryan H Goldstein, Thomas Seery, Karen C Uzark, Anjali Chelliah, Ryan Butts, Heather Henderson, Tiffanie Johnson, Richard V Williams
INTRODUCTION: We sought to evaluate the prevalence of delayed puberty and abnormal anthropometry and its association with quality of life (QoL) in young Fontan survivors. METHODS: This was a cross-sectional study at 11 Pediatric Heart Network centers. Demographic and clinical data, anthropomety, and Tanner stage were collected. Anthropometric measurements and pubertal stage were compared to US norms. QoL was assessed using Pediatric Quality of Life inventory (PedsQL)...
March 9, 2018: Congenital Heart Disease
Melissa G Y Lee, Johnny Millar, Elizabeth Rose, Aleesha Jones, Dora Wood, Taryn L Luitingh, Diana Zannino, Johann Brink, Igor E Konstantinov, Christian P Brizard, Yves d'Udekem
OBJECTIVES: To determine the incidence of vocal cord paresis (VCP) after neonatal aortic arch repair/Norwood-type procedure, and the effectiveness of noninvasive laryngeal ultrasound in detecting VCP compared with gold standard invasive nasoendoscopy. METHODS: Fifty-two patients who underwent an arch repair (39 of 52; 75%) or Norwood-type procedure (13 of 52; 25%) via sternotomy between April 1, 2015, and April 30, 2017 underwent laryngeal ultrasound (50 of 52; 96%) and/or flexible fiber optic nasoendoscopy (39 of 52; 75%) at 48 to 72 hours after endotracheal extubation...
February 9, 2018: Journal of Thoracic and Cardiovascular Surgery
J Lévy, D Haye, N Marziliano, G Casu, F Guimiot, C Dupont, N Teissier, B Benzacken, P Gressens, E Pipiras, A Verloes, A-C Tabet
Ephrin B2, one of the ligand of the EphB receptors, is involved in a complex signaling pathway regulating the development of the nervous system, neuronal migration, erythropoiesis and vasculogenesis. We report a patient with a de novo variant in EFNB2 and a family in which segregates a 610-kb deletion at chromosome 13q33 encompassing only ARGLU1 and EFNB2 genes. The de novo variant was observed in a patient with anal stenosis, hypoplastic left ventricle and mild developmental delay. The deletion was identified in 2 sibs with congenital heart defect and mild developmental delay...
March 6, 2018: Clinical Genetics
Saima Bibi, Syed Yasir Hussain Gilani, Shawana Bibi
BACKGROUND: Congenital heart disease is a significant problem world over especially in neonates. Early diagnosis and prompt interventions in neonatal period precludes the mortality associated with this disorder. The objective of this study was to highlight the diversity of congenital cardiac defects in our region so that appropriate interventions are devised to minimize significant morbidity and mortality associated with this disorder. METHODS: This descriptive cross-sectional study was conducted at the Neonatology Unit of Department of Paediatrics, Ayub Teaching Hospital from January 2015 to December 2016...
January 2018: Journal of Ayub Medical College, Abbottabad: JAMC
Sharon L Cheatham, Joanne L Chisolm, Nicole O'Brien
Hypoplastic left heart syndrome (HLHS) palliation may result in altered cerebral blood flow with subsequent neurodevelopmental implications. The purpose of the study was to assess blood flow in the middle cerebral artery (MCA) and investigate the relationship with early neurodevelopmental outcomes in infants with HLHS after hybrid stage I. Transcranial Doppler (TCD) was performed to obtain peak systolic, end-diastolic, and mean velocities, as well as pulsatility index of the MCA in infants with HLHS (n = 18) at baseline and at 2, 4, and 6 months of age...
March 2, 2018: Pediatric Cardiology
Louise A Kenny, Fabrizio DeRita, Mohamed Nassar, John Dark, Louise Coats, Asif Hasan
The single ventricle patient population comprises the most complex cohort presenting to the cardiac transplant team, in terms of demographics, anatomic substrate, and unique physiology. It is also the most rapidly growing diagnostic group presenting for heart transplantation. In this manuscript, we aim to describe the changing landscape of transplantation in single ventricle conditions through reflection on our own institution's practice and experience, alongside contemporary literature review. Single ventricle patients are heterogeneous in terms of age, anatomic diagnosis and physiology according to surgical stage of repair...
January 2018: Annals of Cardiothoracic Surgery
Hideto Teranishi, Yuhki Koga, Kentaro Nakashima, Eiji Morihana, Kanako Ishii, Yasunari Sakai, Tomoaki Taguchi, Yoshinao Oda, Noriko Miyake, Naomichi Matsumoto, Shouichi Ohga
A 3-year-old Japanese girl treated for hypoplastic left heart syndrome and Dandy-Walker syndrome was diagnosed with Kabuki syndrome (KS) with a mutation of KMT2D; c.13285C>T:p.Q4429*. Concurrently, macrohematuria portended the diagnosis of Wilms tumor. Postoperative chemotherapy has achieved complete remission despite a prolonged and reduced regimen due to liver dysfunction and convulsions. Cancer predisposition has been suggested for KS due to oncogenic mutations in KMT2D or KDM6A. The first case of nephroblastoma exemplified the treatability of malignancies in KS patients, as shown in the 9 cases reviewed...
February 27, 2018: Journal of Pediatric Hematology/oncology
Eva Kapravelou, David Anderson, Gareth J Morgan
The use of hybrid techniques to avoid neonatal cardiopulmonary bypass in high-risk individuals is well reported in the setting of hypoplastic left heart syndrome. We describe the use of that technique as a bridging procedure in high-risk neonates with an interrupted aortic arch. We report three cases where hybrid branch pulmonary artery banding and ductal stent implantation has been successfully used to defer complete repair, allowing recovery, maturity, and weight gain. This strategy may be considered for patients deemed at high risk for primary neonatal repair...
March 2018: International Journal of Angiology: Official Publication of the International College of Angiology, Inc
Kaitlin Balduf, T K Susheel Kumar, Umar Boston, Shyam Sathanandam, Marc V Lee, Tim Jancelewicz, Christopher J Knott-Craig
BACKGROUND: Hypoplastic left heart syndrome (HLHS) is the second most common congenital heart disease associated with congenital diaphragmatic hernia (CDH). The reported survival of neonates with CDH and HLHS is only 1-5%. We review our experience with CDH and HLHS and compare our outcomes to published literature. METHODS: Retrospective review of all neonates with CDH and HLHS at our institution over a 10 year period was performed. The morphology of cardiac and diaphragm defects, clinical course, treatment strategies and outcomes were reviewed and an algorithmic approach is proposed...
February 12, 2018: Seminars in Thoracic and Cardiovascular Surgery
James William Gaynor, Samuel Parry, Julie S Moldenhauer, Rebecca A Simmons, Jack Rychik, Richard F Ittenbach, William W Russell, Erin Zullo, John Laurenson Ward, Susan C Nicolson, Thomas L Spray, Mark P Johnson
OBJECTIVES: Pregnancies with congenital heart disease in the foetus have an increased prevalence of pre-eclampsia, small for gestational age and preterm birth, which are evidence of an impaired maternal-foetal environment (MFE). METHODS: The impact of an impaired MFE, defined as pre-eclampsia, small for gestational age or preterm birth, on outcomes after cardiac surgery was evaluated in neonates (n = 135) enrolled in a study evaluating exposure to environmental toxicants and neuro-developmental outcomes...
February 13, 2018: European Journal of Cardio-thoracic Surgery
Martin Christmann, Emanuela R Valsangiacomo Büchel, Hitendu Dave, Dietrich Klauwer, Anna Cavigelli-Brunner
Background: The period between stage I and II procedure for treatment of hypoplastic left heart syndrome (HLHS) bears high mortality and morbidity. Methods: We sought to analyze the prognostic value of Troponin T/I (Trop), a well-recognized marker for myocardial damage and heart failure, for predicting outcome in a retrospective analysis of 70 infants with HLHS at our institution between March 2001 and October 2014. Results: Stage I procedure consisted of Norwood I operation in 35 (50%) and Hybrid-approach in 22 (31%) patients...
January 2018: Annals of Pediatric Cardiology
Aaron R Prosnitz, Monika Drogosz, Audrey C Marshall, Louise E Wilkins-Haug, Carol B Benson, Lynn A Sleeper, Wayne Tworetzky, Kevin G Friedman
OBJECTIVE: To describe the early hemodynamic changes after fetal aortic valvuloplasty (FAV) for evolving hypoplastic left heart syndrome due to mid-gestational aortic stenosis and to assess whether these early changes predict biventricular (BiV) circulation at neonatal discharge. METHOD: We retrospectively reviewed all technically successful FAV cases resulting in live birth between 2000 and 2015 (n=93, 45% BiV circulation at neonatal discharge). Paired testing methods were used to compare pre- and post-intervention measures of left ventricular hemodynamics...
February 13, 2018: Prenatal Diagnosis
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